临床误诊误治Issue(12)：25-28,4.DOI:10.3969/j.issn.1002-3429.2014.12.009
母细胞性浆细胞样树突细胞肿瘤41例临床分析
Clinical Analysis on 41 Cases of BPDCN
饶进 1殷莉 1林志美 1李世云 1熊萍1
作者信息
- 1. 610081 成都，成都大学附属医院血液内科
- 折叠
摘要
Abstract
Objective To investigate the clinical diagnosis, treatment and prognosis of BPDCN, and to improve the understanding and treatment of BPDCN. Methods Literatures on BPDCN, blastic natural-killer-cell lymphoma or CD4 +CD56+ haematodermic neoplasm published in journals between January 2009 and December 2013 were identified by searching Wanfang, PubMed and CNKI. A retrospective review was carried out. Results A total of 41 BPDCN patients were found from the 28 literatures, including 29 males, 12 females;the median age was 63. 7 years. Major clinical manifestations were skin lesions and often involving lymph nodes and bone marrow. The tumors were CD4 +, CD56 + and CD123 +. Lineage specific markers for B-and T-cell were negative and the tumors did not express myelopemxidase. 33 of the 41 patients accepted lymphoma, acute myeloid leukemia ( AML) , acute Lymphocytic leukemia-like chemothpy and single agent chemotherapy at-tained hematological remission, and 20 patients obtained complete remission. 15 patients were treated with allogeneic SCT, and 14 of the 15 patients obtained hematological remission. The median overall survival ( MS) time of 41cases was 12. 3 months. Conclusion BPDC hematodermic neoplasm is a rare hematopoietic malignancy, which primarily affects elderly pa-tients. The diagnosis of this disease is mainly based on the clinical presentations, pathologic and immunohistochemical fea-tures. Clinical course is rapidly and fatally aggressive. Patients with BPDCN usually have a poor prognosis.关键词
母细胞性浆细胞样树突细胞肿瘤/皮肤表现/免疫表型分型/治疗结果/预后Key words
BPDCN/Skin Manifestations/Immunophenotyping/Treatment outcome/Prognosis分类
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饶进,殷莉,林志美,李世云,熊萍..母细胞性浆细胞样树突细胞肿瘤41例临床分析[J].临床误诊误治,2014,(12):25-28,4.
